Castlemans Disease: a case study

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Castlemans Diseasea case study

• Hematology/Oncology Grand RoundsNovember 8, 2002
• Shao-Chun Chang

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History

• 35-year-old healthy man until July 8, 2002
• Sharp, intense right flank and back pain with
  radiation to groin
• Gross hematuria
• Nausea and vomiting
• No constitutional Sx, no neuro Sx, no other pain,
  no other GU Sx, no HIV risk factors
• No PMHx
• PE RLQ mass with guarding without rebound

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Laboratory

• CBC normal without cytopenia and normal indicies
  and differential
• Chemistry normal
• Urine positive for RBC

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Radiology

• Initial workup for nephrolithiasis
• IVP showed effacement of bladder secondary to a
  pelvic mass
• CT of abdomen/pelvis
• 7.8 x 5 cm mass in right pelvis
• Enlarged mesentery and inguinal lymph nodes
• Mild right hydronephrosis and hydroureter

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Additional Laboratory

• LDH normal
• SPEP
• Polyclonal hypergammaglobulinemia
• Small M-spike in beta region (0.2 g/dl), IgA
  lambda
• Beta2-microglobulin normal
• AFP normal, HCG normal
• HHV8 negative, HIV negative

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Pathology

• Cervical and inguinal biopsy
• Chronic reactive lymphoid hyperplasia
• Pelvic mass biopsy
• Angiofollicular lymphoid hyperplasia, plasma cell
  variant
• Flow cytometry showed polyclonal T and B cell
  populations

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Staging

• Bone marrow biopsy was non-diagnostic
• PET scan
• Uptake to the right pelvic mass and regional
  lymph nodes consistent with CT
• No distant uptake
• Exploratory Laparotomy
• Unresectable pelvic mass encasing the IVC and
  right iliac artery and vein
• Right ureter stent placement

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Castlemans Disease

• Angiofollicular lymphoid hyperplasia
• Giant lymph node hyperplasia
• Angiomatous lymphoid hamartoma
• Lymph nodal hamartoma
• Lymph node hyperplasia of Castleman

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Castlemans Disease

• First described by Benjamin Castleman
• Localized mediastinal lymph-node hyperplasia
  resembling thymoma Cancer (1956), 9822-830
• A series of thirteen cases of mediastinal masses
  resembling thymoma grossly and microscopically
  are shown to be a peculiar form of lymph-node
  hyperplasia characterized by germinal-center
  formation and marked capillary proliferation.
• Evidence is presented that the condition is
  neither neoplastic nor thymic in origin.

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Angiofollicular Lymphoid Hyperplasia
Sickel, J, NEJM (Sept 1995)
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Castlemans Disease Eitology

• Unknown
• Reactive chronic lymphoid hyperplasia
  (Hyaline-vascular type)
• Inflammatory pathogensis (Plasma-cell type)
• Chronic antigenic stimulation (infection)
• Autoimmune mechanism
• Overproduction of IL-6
• Association with HHV8 in 25 multicentric form
• HIV?

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Castlemans Disease

• Keller, AR, Hoghholzer, L, and Castleman, B,
  Cancer (1972), 29670-683
• Case study of 81 patients
• All had localized disease
• Two distinct histologic patterns
• Hyaline-vascular type 74 cases (91)
• Plasma-cell type 7 cases (9)
• Age 8- to 66-years-old
• No gender or race preference

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Castlemans Disease Anatomical Location
Location No. () Neck 5
(6) Mediastinum 70 (86) Anterior-superior 2
5 (31) Middle 3 (4) Posterior 15
(19) Left hilum 9 (11) Right hilum 15
(19) Not specified 3 (4) Intrapulmonary
2 (2) Axilla 1 (1) Retroperitoneal
1 (1) Pelvis 1 (1)
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Castlemans Disease Anatomical Location
Size (greatest diameter) No. () lt 2
cm 1 (1) 2-2.9 cm 4 (5) 3-4.9
cm 25 (31) 5-9.9 cm 42 (52) 10-16 cm
9 (11)
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Angiofollicular Lymphoid Hyperplasia
Hyaline-Vascular Type

• Small follicle center with penetrating
  capillaries
• Interfollicular tissue with small lymphocytes
• Effacement of lymphoid sinsus

Hoffman Hematology
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Angiofollicular Lymphoid Hyperplasia Plasma-Cell
Type

• Large cellular follicle center
• Interfollicular tissue with sheets of plasma
  cells
• Effacement of lymphoid sinsus

Hoffman Hematology
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Castlemans Disease Multicentric

• Histopathologic features of plasma-cell type
• Clinical presentation as a predominantly
  lymphadenopathic disease, consistently involving
  multiple peripheral nodes
• Evidence of multisystem involvement
• Idiopathic

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Castlemans Disease Clinical Symptoms

• Hyaline-vascular type (localized)
• Asymptomatic, except for tumor compression
• Plasma-cell type (localized)
• /- Systemic symptoms
• Plasma-cell type (multicentric)
• By definition with systemic symptom multisystem
  involvement

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Multicentric Castlemans Disease Clinical
Symptoms

• System symptoms (95)
• Malaise 81
• Fever 71
• Weight loss 58
• Night sweats 48
• Anorexia, nausea 42
• Multicentric LAD (100)
• Peripheral 100
• Abdomen 53
• Mediastinum 47

• Splenomegaly 79
• Hepatomegaly 63
• Skin rashes 37
• Neurological Sx
• Central 24
• Peripheral 5
• Kaposis sarcoma 13
• NHL 18

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Multicentric Castlemans Disease Laboratory

• Anemia 89
• Mild (10-13 g/dL) 27
• Moderate-severe (lt10 g/dL) 73
• Leukopenia 21
• Thrombocytopenia 61
• Elevated ESR 95
• Hypergammaglobulinemia 85
• Hypoalbuminemia 100
• Abnormal liver tests 69
• Proteinuria 83

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Castlemans Disease Prognosis and Treatment

• Hyaline-vascular type
• Prognosis good
• Surgical resection
• Plasma-cell type, localized
• Prognosis good
• Curable with local therapy (surgery or radiation)

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Castlemans Disease Prognosis and Treatment

• Plasma-cell type, multicentric
• Unpredictable course (medium survival of 29
  months)
• Cause of death 70 infection and 30 others
  (malignancies, progressive Castlemans disease,
  renal failure)
• Surgical resection
• Radiation
• High dose steroids (1-2 mg/kg/d prednisone)
• Combination chemotherapy (NHL)
• Anti-IL-6 antibodies

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